Since the availability of the H2-receptor antagonists (1978), seven patients admitted to the Department of Emergency Surgery, University of Pisa, with Zollinger-Ellison syndrome (ZES) sustained by extrapancreatic gastrinomas underwent elective surgical treatment. There were four women and three men, with a mean age of 37.3 years (16 to 67 years of age). Preoperative localization studies included endoscopy of the upper part of the gastrointestinal tract, computed axial tomography, ultrasound, selective angiography and transhepatic portal vein sampling for gastrin and were effective in four patients. In the other three patients, the localization of the gastrinoma was made at laparotomy. Four patients had gastrinomas of the duodenal and jejunal wall. Three underwent excisional operations, whereas the remnant was treated with pancreatoduodenectomy. In three of the patients, the location of the gastrinoma was extrapancreatic and extraintestinal, and surgical treatment consisted of nodal excision (two patients) and hepatectomy. None of the patients had undergone a gastric operation in addition to tumorectomy procedures. Soon after excisional operation, ZES recurred in one patient and a second laparotomy with nodal excision resulted in a cure. On long term follow-up evaluation, ranging from 15 to 136 months (mean of 85.1 divided by 37.3 months), no patients showed a recurrence of ZES. The possibility of extrapancreatic gastrinoma should be suspected in patients with sporadic ZES. The need for emergency surgical treatment in these patients is minimized by the medical control of the gastric secretion that consents diagnostic studies for the preoperative localization of the gastrinoma. Even if only suspected, the site of the extrapancreatic gastrinoma has an increased chance to be identified at operation. This consents to remove gastrinomas mostly by a minimal excisional procedure resulting in the normalization of gastric secretion and the prevention of malignant evolution of these tumors.